Ua lohe paha ʻoe i ka "anemia" a i ʻole "anemia" ʻeā? I ka ʻōlelo maʻalahi, ʻo ia ka wā ʻaʻole lawa ka hana ʻana o ko mākou kino i nā hunaola koko ʻulaʻula a i ʻole ʻaʻole hana pono nā hunaola koko ʻulaʻula i loaʻa iā mākou. ʻO nā hunaola koko ʻulaʻula nā mea lawe nui o ka oxygen ma o ko mākou kino. No laila, ke haʻahaʻa lākou, hiki iā mākou ke luhi a palaualelo i nā manawa a pau. Hana ʻia kēia mau hunaola koko ʻulaʻula i loko o ka iwi iwi, kahi ʻiʻo palupalu a huʻahuʻa i loko o ko mākou mau iwi. ʻO Diamond-Blackfan Anemia (DBA) kahi ʻano anemia kūikawā e kū mai ana i ka wā ʻaʻole hiki i ka iwi iwi ke hana i nā hunaola koko ʻulaʻula lawa.
He aha ke kumu o ka Diamond-Blackfan Anemia (DBA)?
ʻO ka Diamond-Blackfan Anemia, i kapa ʻia i kekahi manawa ʻo "acquired pure red cell aplasia", ʻike pinepine ʻia e nā kauka ma mua o ka piha ʻana o kahi keiki i hoʻokahi makahiki. ʻO ke kumu nui o kēia, ʻo ia nā loli, a i ʻole nā mutations, i loko o nā genes ʻo ia nā poloka kūkulu o ko mākou kino.
E hoʻomanaʻo, i kekahi manawa hiki ke hoʻoilina ʻia kēia kīnā genetic mai ka makuahine a i ʻole ka makuakāne. Akā, ʻaʻole ia ka hihia mau. Hiki i kekahi mau genes o nā keiki ke loli koke me ke kumu ʻole. ʻO ka hapa nui o nā keiki me DBA i loaʻa i kēia maʻi ma muli o kahi hoʻololi genetic hou. No laila, ma ke ʻano he mau mākua, ʻaʻohe pono e hopohopo pono ʻole e pili ana i kēia. ʻOiai ua loaʻa kahi kumu genetic kikoʻī no ka hapalua o nā maʻi DBA, no kekahi poʻe, ʻaʻole i loaʻa kahi kumu kikoʻī no kēia maʻi.
He aha nā hōʻailona o DBA?
Hiki i nā keiki me DBA ke ʻike i nā hōʻailona maʻamau o nā ʻano anemia ʻē aʻe. ʻO ia hoʻi,
- Luhi mau
- ʻIli keʻokeʻo
- Manaʻo nāwaliwali
Ma waho aʻe o kēia mau hiʻohiʻona maʻamau, hiki i kekahi mau keiki i hānau ʻia me DBA ke loaʻa nā hiʻohiʻona waho kūikawā ma ko lākou mau helehelena a me ke kino. E nānā kākou i ke ʻano o lākou.
| ʻĀpana kino | Nā hiʻohiʻona i ʻike ʻia |
|---|---|
| Poʻo | He poʻo liʻiliʻi ma mua o ka maʻamau. |
| Maka | Hoʻonui ʻia ka mamao ma waena o nā maka a me ka ihu pālahalaha. |
| Nā pepeiao | ʻElua mau pepeiao liʻiliʻi i hoʻonoho ʻia ma lalo o ka maʻamau. |
| ʻAuwae | He ʻāwae liʻiliʻi ko lalo. |
| ʻĀʻī | He ʻāʻī pōkole a ulana ʻia. |
| Nā poʻohiwi | Nā ʻūpā poʻohiwi liʻiliʻi. |
| Nā lima | Nā manamana nui i ke ʻano ʻē. |
| Waha | Loaʻa i kahi lehelehe māwae a i ʻole ka lehelehe māwae. |
Eia kekahi, hiki i ka DBA ke hoʻoulu i nā pilikia puʻupaʻa , nā kīnā o ka puʻuwai , a me nā pilikia maka e like me ke cataracts a me ka glaucoma . Hiki i nā keikikāne ke loaʻa i kahi kīnā hānau i kapa ʻia ʻo hypospadias, ʻo ia hoʻi ka wā ʻaʻole ka puka no ka mimi ma ka piko o ka penis.
Pehea e ʻike pono ai i kēia maʻi?
Hiki i kāu kauka ke hana i kekahi mau hoʻokolohua e hoʻoholo ai inā loaʻa i kāu keiki ka DBA. He haʻahaʻa ka helu o nā cell koko ʻulaʻula o nā keiki me ka DBA, akā he maʻamau ka helu o nā cell koko keʻokeʻo a me nā platelet.
ʻO ka mea nui loa, ʻaʻole e hopohopo ke ʻike ʻoe i kēia mau hōʻailona, akā e ʻike koke i ke kauka kūpono. E hana ʻo ia i nā hoʻokolohua e pono ai a hāʻawi iā ʻoe i ka ʻike pololei loa.
ʻO kēia kekahi o nā hoʻokolohua nui i hana ʻia e ke kauka.
| Hoʻāʻo | He aha kāna mea e nānā ai? |
|---|---|
| Helu Koko Piha (CBC) | Nui nā mea i ana ʻia i ke koko, e like me ka helu o nā hunaola koko ʻulaʻula, nā hunaola koko keʻokeʻo, nā platelets, ka hemoglobin protein lawe oxygen, a me ka nui o nā hunaola koko ʻulaʻula (hematocrit) . |
| Helu o nā reticulocyte | Ana kēia i ka helu o nā hunaola koko ʻulaʻula i ʻōpiopio, a i ʻole i hana hou ʻia. Hiki i kēia ke haʻi inā ke hana pono nei ka iwi iwi i nā hunaola koko ʻulaʻula. |
| Ke nānā nei i ka pae eADA | Ma kahi o 80% o ka poʻe me DBA he kiʻekiʻe ko lākou pae o kahi enzyme i kapa ʻia ʻo erythrocyte adenosine deaminase (eADA) . ʻO kēia ka mea e nānā ʻia nei e kēia hoʻokolohua. |
| Nā pae hemoglobin o ka pēpē | ʻO kēia ke ʻano o ka hemoglobin i loaʻa i nā pēpē i loko o ka ʻōpū. Pono e emi iho ma hope o ka hānau ʻana. Eia nō naʻe, hiki i nā keiki me DBA ke loaʻa mau nā pae kiʻekiʻe i ko lākou ulu ʻana. |
| Ka makemake ʻana i ka iwi iwi a me ka biopsy | Lawe ʻia kahi liʻiliʻi o nā pūnaewele a me ka wai mai ka iwi iwi me ka hoʻohana ʻana i kahi nila a hoʻāʻo ʻia. He kakaikahi loa nā hunaola koko ʻulaʻula olakino o nā keiki me DBA i loko o ko lākou iwi iwi. |
| Nā hoʻokolohua genetic | Hoʻāʻo kēia i nā genes e pili ana i ka DBA a i ʻole nā anemia ʻē aʻe i hoʻoilina ʻia mai nā mākua mai. |
He aha nā pilikia ʻē aʻe e hiki ke hana ma muli o DBA?
ʻOi aku ka nui o ka pilikia o ka poʻe me DBA i ka loaʻa ʻana o kekahi mau maʻi a me nā kūlana. ʻO kēia mau mea nā maʻi kanesa o ke koko a me ka iwi iwi (acute myeloid leukemia), ka maʻi kanesa o ka iwi (osteosarcoma), a me nā maʻi ʻē aʻe kahi i hiki ʻole ai i ka iwi iwi ke hana i nā hunaola koko olakino (myelodysplastic syndrome). Akā, mai hopohopo no kēia. Ke nānā mau nei nā kauka i kēia, no laila hiki iā ʻoe ke lawe i nā hana lapaʻau e pono ai i ka manawa kūpono.
He aha nā lāʻau lapaʻau no kēia?
ʻO kēia ka ʻāpana koʻikoʻi a hōʻoluʻolu hoʻi. Hiki i nā keiki i ʻike ʻia me DBA ke ola lōʻihi a holomua me ka mālama ʻana i ka lāʻau lapaʻau. Hele kekahi mau keiki i ke kala piha ʻana, ʻo ia hoʻi, nalowale nā hōʻailona no kekahi manawa.
ʻO nā lāʻau lapaʻau nui ʻelua i hoʻohana ʻia, ʻo ia nā lāʻau corticosteroid a me nā hoʻoili koko.
- Nā lāʻau Corticosteroid: Hoʻoulu nā lāʻau e like me '(Prednisone)' i ka iwi iwi a kōkua iā ia e hana i nā hunaola koko ʻulaʻula hou aʻe.
- Hoʻoili koko: He koho maikaʻi kēia inā ʻaʻole maikaʻi nā lāʻau steroid, a inā koʻikoʻi ka anemia. Pili kēia i ka hāʻawi ʻana i ke keiki i ke koko a i ʻole nā kelepona koko ʻulaʻula mai kahi mea hāʻawi olakino.
- Hoʻoili iwi/pūnaewele kumu: ʻO kēia wale nō ka lāʻau lapaʻau no DBA. Eia nō naʻe, he mea pilikia iki ia. Eia kekahi, hiki ke paʻakikī i kekahi manawa ka loaʻa ʻana o kahi mea hāʻawi kūpono.
He mea nui e kūkākūkā pono i kēia mau koho lapaʻau a pau, ko lākou mau pono a me nā pōʻino, me kāu kauka, a koho i ka mea maikaʻi loa no kāu keiki.
Leka Lawe-Home
- ʻO ka Diamond-Blackfan Anemia (DBA) kahi maʻi genetic kakaikahi i nā keiki kahi i hiki ʻole ai i ka iwi iwi ke hana i nā hunaola koko ʻulaʻula lawa.
- Me nā hōʻailona maʻamau e like me ka luhi pinepine a me ka maeʻele, hiki i kēia maʻi ke hana i kekahi mau hiʻohiʻona waho kikoʻī ma ka maka a me ke kino.
- Hiki i ke kauka ke ʻike pololei i kēia maʻi ma o nā hoʻāʻo koko a me ka hoʻokolokolo ʻana i ka iwi iwi.
- Hiki i nā lāʻau lapaʻau e like me nā lāʻau steroid a me nā hoʻoili koko ke kōkua i nā keiki e ola lōʻihi a olakino. ʻO ka hoʻoili iwi iwi wale nō ka lāʻau lapaʻau e ho'ōla ai.
- Inā loaʻa iā ʻoe kekahi kānalua iki ua loaʻa i kāu keiki kēia mau hōʻailona, mai hopohopo a e ʻike koke i kahi kauka keiki. He mea nui loa ka ʻike wikiwiki a me ka mālama ʻana.
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